SCLERODERMA AND A COMPLICATED CASE OF HYPERTENSIVE CRISIS, RENAL FAILURE AND RESPIRATORY FAILURE: A CASE REPORT

Imran Khan*, Bernadett Kovacs1, Marlene Mansour, Andy Pan, Caitlin Richler, Christine Landry, Sydney Morin, Kaouther Derouiche, and Pierre Thabet,

ABSTRACT

Background: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis characterized by abrupt malignant hypertension, rapidly progressive acute kidney injury, and microangiopathic hemolysis. Although traditionally associated with diffuse cutaneous disease, patients with limited cutaneous systemic sclerosis and high-risk serologic profiles—particularly RNA polymerase III antibodies—remain at substantial risk. Early recognition and uninterrupted renin–angiotensin–aldosterone system (RAAS) blockade are critical to improving outcomes, yet management is often complicated by overlapping cardiopulmonary disease and hemodynamic instability. Objective: To describe a complex case of scleroderma renal crisis presenting with hypertensive emergency, acute renal failure, and respiratory failure in a patient with limited systemic sclerosis and RNA polymerase III antibody positivity, highlighting diagnostic challenges and pathophysiology-guided management. Case Presentation: A 68-year-old man with limited systemic sclerosis, severe chronic obstructive pulmonary disease, and strongly positive RNA polymerase III antibodies presented with acute respiratory distress, malignant hypertension (217/143 mmHg), and severe acute kidney injury (creatinine 486 μmol/L; baseline 103 μmol/L). Initial management addressed respiratory failure and possible infection; however, evolving microangiopathic hemolysis and persistent severe hypertension shifted the working diagnosis toward SRC. Intravenous enalaprilat was initiated and transitioned to high-dose oral captopril with rapid titration. Blood pressure remained markedly labile, requiring multimodal antihypertensive therapy including nitrates, hydralazine, and calcium channel blockade. Renal function worsened despite RAAS inhibition, and brief continuous renal replacement therapy (CRRT) was required before interruption. Aggressive diuresis subsequently improved pulmonary edema and respiratory status. Renal recovery remained incomplete at discharge planning. Conclusions: This case underscores the diagnostic complexity of SRC in patients with limited cutaneous systemic sclerosis and high-risk serology. Extreme blood pressure lability, overlapping cardiopulmonary disease, and apparent hypovolemia can obscure early recognition. Prompt initiation and continuation of short-acting ACE inhibitors, even in the setting of rising creatinine, remain central to management. Multidisciplinary coordination and pathophysiology-driven hemodynamic strategies are essential in stabilizing this high-risk phenotype.

Keywords: Systemic Sclerosis; Scleroderma Renal Crisis; RNA Polymerase III Antibodies; Hypertensive Emergency; Acute Kidney Injury; Microangiopathic Hemolysis; Renin–Angiotensin System; Critical Care; Case Report; Multisystem Disease.